This study evaluated the myocardial strain and aortic elasticity in patients with bicuspid aortic valve (BAV) and then investigated the relation between them. Thirty-nine patients (30 males; mean age 44±19 years; range 6 to 75 years) with BAV were recruited as BAV group, and 29 age- and sex-matched healthy controls (21 males; mean age 42±11 years; range 20 to 71 years) served as control group. Aortic strain, distensibility and stiffness index were derived using M-mode echocardiography. Left ventricular global myocardial strain was acquired with speckle-tracking echocardiography. Correlation between aortic elasticity and myocardial strain was also analyzed. The results showed that aortic stiffness was higher (17.5±14.0 vs. 5.3±2.7, P<0.001), and aortic strain (4.9±4.7 vs. 11.0±4.1, P<0.001) and distensibility (1.8±2.1 vs. 3.7±1.6, P<0.001) were lower significantly in BAV group than in control group. Global circumferential strain (-19.1±4.2 vs.-22.5±3.7, P<0.001), radial stain (29.8±14.9 vs. 38.0±8.8, P<0.001) and longitudinal stain (-18.4±3.4 vs.-20.8±3.5, P<0.001) were significantly lower in BAV group than in control group. There was weak association between aortic elasticity and myocardial strain. These findings indicated BAV patients manifest reduced myocardial strain which had weak relationship with aortic elastic lesion.
Adolescent ; Adult ; Aged ; Aorta ; pathology ; Aortic Valve ; abnormalities ; pathology ; Child ; Echocardiography ; Elasticity ; Female ; Heart Valve Diseases ; pathology ; Humans ; Male ; Middle Aged ; Myocardium ; pathology ; Sprains and Strains ; pathology ; Vascular Stiffness ; physiology

With the population aging and declining incidence of rheumatic heart disease, calcific aortic valve disease (CAVD) has become the most frequent valve disease and the common cause of aortic valve replacement. Patients with CAVD need to cope with a deteriorating quality of life and valve replacement is the only effective clinical option for the patients. Therefore, early pharmacotherapy is of great significance in prevention or slow-down of the progression of CAVD. For years CAVD was considered to be a passive wear and tear process of valves, but now it is recognized as an active and multi-factorial process. Histopathologic studies have revealed that inflammation, disorder of calcium and phosphorus metabolism and dyslipidemia are involved in the process of CAVD. Clinical trials of CAVD pharmacotherapy have been carried out based on those histopathologic studies. Statin, renin-angiotensin inhibitors and anti-osteoporosis drug are well studied in recent years. This article reviews the recent research progress of the pharmacotherapy for CAVD.
Angiotensin Receptor Antagonists ; therapeutic use ; Angiotensin-Converting Enzyme Inhibitors ; therapeutic use ; Aortic Valve ; pathology ; Aortic Valve Stenosis ; complications ; drug therapy ; etiology ; Calcinosis ; complications ; drug therapy ; etiology ; Calcium Metabolism Disorders ; complications ; Disease Progression ; Dyslipidemias ; complications ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; therapeutic use ; Inflammation ; complications ; Phosphorus Metabolism Disorders ; complications ; Quality of Life

BACKGROUND: Although unique aortic pathology related to bicuspid aortic valve (BAV) has been previously reported, clinical implications of BAV to aortopathy risk have yet to be investigated. We looked for potential differences in matrix protein expressions in the aortic wall in BAV patients. METHODS: Aorta specimens were obtained from 31 patients: BAV group (n=27), tricuspid aortic valve (TAV) group (n=4). The BAV group was categorized into three subgroups: left coronary sinus-right coronary sinus (R+L group; n=13, 42%), right coronary sinus-non-coronary sinus (R+N group; n=8, 26%), and anteroposterior (AP group; n=6, 19%). We analyzed the expression of endothelial nitric oxide synthase (eNOS), matrix metalloproteinase (MMP)-9, and tissue inhibitor of matrix metalloproteinase (TIMP)-2. RESULTS: Based on the mean value of the control group, BAV group showed decreased expression of eNOS in 72.7% of patients, increased MMP-9 in 82.3%, and decreased TIMP in 79.2%. There was a higher tendency for aortopathy in the BAV group: eNOS (BAV:TAV)= 53%±7%:57%±11%, MMP-9 (BAV:TAV)=48%±10%:38%±1%. The AP group showed lower expression of eNOS than the fusion (R+L, R+N) group did; 48%±5% vs. 55%±7% (p=0.081). CONCLUSION: Not all patients with BAV had expression of aortopathy; however, for patients who had a suspicious form of bicuspid valve, aortic wall biopsy could be valuable to signify the presence of aortopathy.
Aorta ; Aortic Valve* ; Bicuspid* ; Biopsy* ; Coronary Sinus ; Humans ; Matrix Metalloproteinase 9 ; Mitral Valve* ; Nitric Oxide Synthase Type III ; Pathology* ; Tissue Inhibitor of Metalloproteinase-2

PURPOSE: In this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockade therapy in treatment of Marfan aortopathy. MATERIALS AND METHODS: We reviewed Marfan syndrome (MFS) patients who underwent aortic root replacement (ARR) between January 1996 and January 2011. All patients were prescribed beta-blockers indefinitely. We compared major aortic events including mortality, aortic dissection, and reoperation in patients without RAAS blockade (group 1, n=27) to those with (group 2, n=63). The aortic growth rate was calculated by dividing the diameter change on CT scans taken immediately post-operatively and the latest scan available. RESULTS: There were no differences in clinical parameters except for age which was higher in patients with RAAS blockade. In group 1, 2 (7%) deaths, 5 (19%) aortic dissections, and 7 (26%) reoperations occurred. In group 2, 3 (5%) deaths, 2 (3%) aortic dissections, and 3 (5%) reoperations occurred. A Kaplan-Meier plot demonstrated improved survival free from major aortic events in group 2. On multivariate Cox, RAAS blockade was an independent negative predictor of major aortic events (hazard ratio 0.38, 95% confidence interval 0.30-0.43, p=0.002). Mean diameter change in descending thoracic and supra-renal abdominal aorta was significantly higher in patients without RAAS blockade (p<0.05). CONCLUSION: In MFS patients who underwent ARR, the addition of RAAS blockade to beta-blocker was associated with reduction of aortic dilatation and clinical events.
Adrenergic beta-Antagonists/pharmacology ; Aged ; Aneurysm, Dissecting/complications/mortality/surgery ; *Angiotensin Receptor Antagonists ; Angiotensin-Converting Enzyme Inhibitors/*therapeutic use ; Aorta/pathology/*surgery ; Aortic Aneurysm/complications/mortality/surgery ; Aortic Valve ; Female ; Humans ; Male ; Marfan Syndrome/mortality/*surgery ; Middle Aged ; Renin-Angiotensin System/*drug effects

A 21-year-old woman with a history of systemic lupus erythematosus (SLE) was admitted with dyspnea on exertion for a year. A transesophageal echocardiogram showed dilated aortic root with intimal thickening. A positron emission tomography/computed tomography demonstrated increase in glucose hypermetabolic along the walls of the aortic valve, ascending aorta, aortic arch, and aorta, vasculitis was observed. She underwent the Bentall operation due to inflammation at sinus of right coronary cusp. She started high dose glucocorticoid after the operation. Currently she is able to sustain with low dose steroid after gradually tapered. Her symptoms were disappeared, and inflammatory markers decreased to within the normal range. Aortitis and aortic aneurysms are an uncommon manifestation of SLE. Furthermore, almost of lupus patients with medium and large vessel vasculitis are not biopsied or studied histologically. We present first case in Korea that was a 21-year-old woman who diagnosed with lupus aortitis by pathology after aortic valve replacement operation.
Aorta ; Aorta, Thoracic ; Aortic Aneurysm ; Aortic Valve ; Aortitis* ; Dyspnea ; Electrons ; Female ; Glucose ; Humans ; Inflammation ; Korea ; Lupus Erythematosus, Systemic* ; Pathology ; Reference Values ; Vasculitis ; Young Adult

Aged ; Aortic Valve ; pathology ; surgery ; Endocarditis ; complications ; surgery ; Endocarditis, Bacterial ; complications ; surgery ; Female ; Glomerulonephritis ; etiology ; Humans

BACKGROUNDThis study was to investigate the relationship among aortic artery calcification (AAC), cardiac valve calcification (CVC), and mortality in maintenance hemodialysis (MHD) patients.

METHODSAll MHD patients in Shanghai Ruijin Hospital in July 2011 were included. To follow up for 42 months, clinical data, predialysis blood tests, echocardiography, and lateral lumbar X-ray plain radiography results were collected. Plasma FGF23 level was measured using a C-terminal assay.

RESULTSTotally, 110 MHD patients were involved in this study. Of which, 64 (58.2%) patients were male, the mean age was 55.2 ± 1.4 years old, and the median dialysis duration was 29.85 (3.0-225.5) months. About 25.5% of the 110 MHD patients had CVC from echocardiography while 61.8% of the patients had visible calcification of aorta from lateral lumbar X-ray plain radiography. After 42 months follow-up, 25 (22.7%) patients died. Kaplan-Meier analysis showed that patients with AAC or CVC had a significant greater number of all-cause and cardiovascular deaths than those without. In multivariate analyses, the presence of AAC was a significant factor associated with all-cause mortality (hazard ratio [HR]: 3.149, P = 0.025) in addition to lower albumin level and lower 25-hydroxy Vitamin D (25(OH)D) level. The presence of CVC was a significant factor associated with cardiovascular mortality (HR: 3.800, P = 0.029) in addition to lower albumin level and lower 25(OH)D level.

CONCLUSIONLateral lumbar X-ray plain radiography and echocardiography are simple methods to detect AAC and CVC in dialysis patients. The presence of AAC and CVC was independently associated with mortality in MHD patients. Regular follow-up by X-ray and echocardiography could be a useful method to stratify mortality risk in MHD patients.

Aortic Diseases ; blood ; complications ; Calcinosis ; blood ; complications ; China ; Female ; Fibroblast Growth Factors ; blood ; Follow-Up Studies ; Heart Valve Diseases ; blood ; complications ; Heart Valves ; pathology ; Humans ; Male ; Middle Aged ; Proportional Hazards Models ; Renal Dialysis ; mortality

Aortic valve calcification is a common disease in the elderly, but its cellular and molecular mechanisms are not clear. In order to verify the hypothesis that Wnt/β-catenin signaling pathway is involved in the process of calcification of aortic valve, porcine aortic valve interstitial cells (VICs) were isolated, cultured and stimulated with oxidized low density lipoprotein (ox-LDL) for 48 h to induce the differentiation of VICs into osteoblast-like cells. The key proteins and genes of Wnt/β-catenin signaling pathway, such as glycogen synthase kinase 3β (GSK-3β) and β-catenin, were detected by using Western blotting and real-time polymerase chain reaction (PCR). The results showed that the VICs managed to differentiate into osteoblast-like cells after the stimulation with ox-LDL and the levels of proteins and genes of GSK-3β and β-catenin were increased significantly in VICs after stimulation for 48 h (P<0.05). It is suggested that Wnt/β-catenin signaling pathway may play a key role in the differentiation of VICs into osteoblast-like cells and make great contribution to aortic valve calcification.
Alkaline Phosphatase ; genetics ; metabolism ; Animals ; Aortic Valve ; metabolism ; pathology ; Aortic Valve Stenosis ; Blotting, Western ; Bone Morphogenetic Protein 2 ; genetics ; metabolism ; Calcinosis ; Cell Differentiation ; drug effects ; genetics ; Cells, Cultured ; Gene Expression ; drug effects ; Glycogen Synthase Kinase 3 ; genetics ; metabolism ; Lipoproteins, LDL ; pharmacology ; Osteoblasts ; drug effects ; metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; Swine ; Wnt Signaling Pathway ; genetics ; physiology ; beta Catenin ; genetics ; metabolism

Aortic Valve ; pathology ; Aortic Valve Stenosis ; genetics ; Calcinosis ; genetics ; Humans

Aortic Valve Stenosis ; blood ; diagnosis ; etiology ; Child ; Coronary Artery Disease ; blood ; diagnosis ; etiology ; Coronary Vessels ; diagnostic imaging ; pathology ; Echocardiography ; Female ; Humans ; Hyperlipoproteinemia Type II ; blood ; complications ; diagnosis ; genetics ; Lipoproteins, LDL ; blood ; Triglycerides ; blood